Guidelines for the management of

by the comprehensive care team and should 

only be initiated after adequate education and 

training. (Level 3) [36,37]

GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA

14

6.  Teaching should focus on general knowledge of 

hemophilia; recognition of bleeds and common 

complications; first aid measures; dosage calcula-

tion; preparation, storage, and administration of 

clotting factor concentrates; aseptic techniques; 

performing venipuncture (or access of central 

venous catheter); record keeping; proper storage 

and disposal of needles/sharps; and handling of 

blood spills. A certification program is helpful.

7.  Patients or parents should keep bleed records 

(paper or electronic) that include date and site 

of bleeding, dosage and lot number of product 

used, and adverse effects. 

8.  Infusion technique and bleed records should be 

reviewed and monitored at follow-up visits.

9.  Home care can be started with young children 

with adequate venous access and motivated family 

members who have undergone adequate training. 

Older children and teenagers can learn self-infu-

sion with family support.

10. An implanted venous access device (Port-A-

Cath) can make injections much easier and may 

be required for administering prophylaxis in 

younger children. (Level 2) [39,40]

11. However, the risks of surgery, local infection, 

and thrombosis associated with such devices 

need to be weighed against the advantages of 

starting intensive prophylaxis early. (Level 2) 

[41,42]

12. The venous access device must be kept scrupu-

lously clean and be adequately flushed after each 

administration to prevent clot formation [41]. 

1.8  Monitoring health status and outcome 

1.  Regular standardized evaluation at least every 

12 months allows longitudinal assessment for 

individual patients and can identify new or 

potential problems in their early stages so that 

treatment plans can be modified. (Level 3) 

[14,26,43]

2.  Patients should be seen by the multidisciplinary 

care team after every severe bleeding episode.

3.  The following should be evaluated and education 

should be reviewed and reinforced:

 ■ issues related to venous access

 ■ issues related to hemostasis (bleed record)

 ■ use of products for replacement therapy and 

the response to them 

 ■ musculoskeletal status: impairment and 

function through clinical assessment of joints 

and muscles, and radiological evaluation 

annually or as indicated (see ‘Musculoskeletal 

complications’, on page 55)

 ■ transfusion-transmitted infections: commonly 

HIV, HCV, and HBV, and others if indicated (see 

‘Transfusion-transmitted and other infection-

related complications’, on page 61)

 ■ development of inhibitors (see ‘Inhibitors’, on 

page 59)

 ■ overall psychosocial status 

 ■ dental/oral health 

4.  Several hemophilia-specific scores are available to 

measure joint impairment and function, including 

activities and participation. These include: 

 ■ Impairment: 

 ■

Clinical: WFH Physical Examination Score 

(aka Gilbert score), Hemophilia Joint 

Health Score (HJHS) 

 ■

Radiological: Pettersson score, MRI, and 

ultrasound scores 

 ■

Activity: Haemophilia Activities List 

(HAL), Paediatric Haemophilia Activities 

List (PedHAL), Functional Independence 

Score in Hemophilia (FISH) 

 ■ Health-related quality of life: (HaemoQol, 

Canadian Hemophilia Outcomes: Kids’ Life 

Assessment Tool [CHO-KLAT])

5.  For more information on available func-

tional and physical examination scores, see the 

WFH’s Compendium of Assessment Tools at: 

www.wfh.org/assessment_tools. 

GENERAL CARE AND MANAGEMENT OF HEMOPHILIA 

15

1.9  Pain management 

1.  Acute and chronic pain are common in patients 

with hemophilia. Adequate assessment of 

the cause of pain is essential to guide proper 

management.

Pain caused by venous access

1.  In general, no pain medication is given.

2.  In some children, application of a local anes-

thetic spray or cream at the site of venous access 

may be helpful. 

Pain caused by joint or muscle bleeding

1.  While clotting factor concentrates should be 

administered as quickly as possible to stop 

bleeding, additional drugs are often needed for 

pain control (see Table 1-5: Strategies for pain 

management in patients with hemophilia). 

2.  Other measures include cold packs, immobili-

zation, splints, and crutches [44].

Post-operative pain

1.  Intramuscular injection of analgesia should be 

avoided.

2.  Post-operative pain should be managed in coor-

dination with the anesthesiologist.

3.  Initially, intravenous morphine or other narcotic 

analgesics can be given, followed by an oral opioid 

such as tramadol, codeine, hydrocodone, and 

others. 

4.  When pain is decreasing, paracetamol/acetamin-

ophen may be used. 

Pain due to chronic hemophilic arthropathy

1.  Chronic hemophilic arthropathy develops in 

patients who have not been adequately treated 

with clotting factor concentrates for joint 

bleeding.

2.  Treatment includes functional training, adap-

tations, and adequate analgesia as suggested in 

Table 1-5. (Level 2) [15,45]

3.  COX-2 inhibitors have a greater role in this 

situation. (Level 2) [46,47]

4.  Other NSAIDs should be avoided. (Level 2) [48]

5.  When pain is disabling, orthopedic surgery 

may be indicated. (Level 5) [49]

6.  Patients with persisting pain should be referred 

to a specialized pain management team.

TABLE 1-5: STRATEGIES FOR PAIN MANAGEMENT IN PATIENTS WITH HEMOPHILIA

1

Paracetamol/acetaminophen 

If not effective

2

COX-2 inhibitor (e.g. celecoxib, meloxicam, nimesulide, and others)

OR

Paracetamol/acetaminophen plus codeine (3-4 times/day)

OR

Paracetamol/acetaminophen plus tramadol (3-4 times/day)

3

Morphine: use a slow release product with an escape of a rapid release.  

Increase the slow release product if the rapid release product is used more than 4 times/day

Notes: 

 

■ If for any reason medications have been stopped for a period of time, patients who have been taking and tolerating high-dose narcotic 

drugs should re-start the drug at a lower dose, or use a less powerful painkiller, under the supervision of a physician.

 

■ COX-2 inhibitors should be used with caution in patients with hypertension and renal dysfunction.

GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA

16

1.10  Surgery and invasive procedures

1.  Surgery may be required for hemophilia-related 

complications or unrelated diseases. The following 

issues are of prime importance when performing 

surgery on persons with hemophilia.

2.  Surgery for patients with hemophilia will require 

additional planning and interaction with the 

healthcare team than what is required for other 

patients. 

3.  A hemophilia patient requiring surgery is best 

managed at or in consultation with a compre-

hensive hemophilia treatment centre. (Level 3) 

[50,51]

4.  The anesthesiologist should have experience 

treating patients with bleeding disorders.

5.  Adequate laboratory support is required for 

reliable monitoring of clotting factor level and 

inhibitor testing.

6.  Pre-operative assessment should include inhib-

itor screening and inhibitor assay, particularly 

if the recovery of the replaced factor is signif-

icantly less than expected. (Level 4) [52,53]

7.  Surgery should be scheduled early in the week 

and early in the day for optimal laboratory and 

blood bank support, if needed. 

8.  Adequate quantities of clotting factor concen-

trates should be available for the surgery itself 

and to maintain adequate coverage post-opera-

tively for the length of time required for healing 

and/or rehabilitation. 

9.  If clotting factor concentrates are not available, 

adequate blood bank support for plasma compo-

nents is needed.

10. The dosage and duration of clotting factor 

concentrate coverage depends on the type of 

surgery performed (see Tables 7-1 and 7-2).

TABLE 1-6: DEFINITION OF ADEQUACY OF HEMOSTASIS FOR SURGICAL PROCEDURES [64]

Excellent

Intra-operative and post-operative blood loss similar (within 10%) to the non-hemophilic patient.

 

■ No extra (unplanned) doses of FVIII/FIX/bypassing agents needed AND

 

■ Blood component transfusions required are similar to non-hemophilic patient

Good

Intra-operative and/or post-operative blood loss slightly increased over expectation for the non-hemophilic patient 

(between 10-25% of expected), but the difference is judged by the involved surgeon/anaesthetist to be clinically 

insignificant.

 

■ No extra (unplanned) doses of FVIII/FIX/bypassing agents needed AND

 

■ Blood component transfusions required are similar to the non-hemophilic patient

Fair

Intra-operative and/or post-operative blood loss increased over expectation (25-50%) for the non-hemophilic 

patient and additional treatment is needed.

 

■ Extra (unplanned) dose of FVIII/FIX/bypassing agents needed OR

 

■ Increased blood component (within 2 fold) of the anticipated transfusion requirement 

Poor/none 

Significant intra-operative and/or post-operative blood loss that is substantially increased over expectation (>50%) 

for the non-hemophilic patient, requires intervention, and is not explained by a surgical/medical issue other than 

hemophilia

 

■ Unexpected hypotension or unexpected transfer to ICU due to bleeding OR

 

■ Substantially increased blood component (> 2 fold) of the anticipated transfusion requirement

Notes:

 

■ Apart from estimates of blood loss during surgery, data on pre- and post-operative hemogloblin levels and the number of packed red 

blood cell units transfused may also be used, if relevant, to estimate surgical blood loss. 

 

■ Surgical hemostasis should be assessed by an involved surgeon and/or anaesthetist and records should be completed within 72 hours 

following surgery. 

 

■ Surgical procedures may be classified as major or minor. A major surgical procedure is defined as one that requires hemostatic support 

for periods exceeding 5 consecutive days.

GENERAL CARE AND MANAGEMENT OF HEMOPHILIA 

17

11. Effectiveness of hemostasis for surgical proce-

dures may be judged as per criteria defined by 

the Scientific and Standardization Committee 

of the International Society on Thrombosis and 

Haemostasis (see Table 1-6) [64].

12. Patients with mild hemophilia A, as well as 

patients receiving intensive factor replacement 

for the first time, are at particular risk of inhib-

itor development and should be re-screened 

4–12 weeks post-operatively. (Level 4) [54]

13. Careful monitoring for inhibitors is also advis-

able in patients with non-severe hemophilia A 

receiving continous infusion after surgery [55]. 

14. Infusion of factor concentrates/hemostatic agents 

is necessary before invasive diagnostic proce-

dures such as lumbar puncture, arterial blood 

gas determination, or any endoscopy with biopsy.

1.11  Dental care and management 

1.  For persons with hemophilia, good oral hygiene is 

essential to prevent periodontal disease and dental 

caries, which predispose to gum bleeding [56]. 

2.  Dental examinations should be conducted regu-

larly, starting at the time the baby teeth start to 

erupt.

3.  Teeth should be brushed twice a day with a 

medium texture brush to remove plaque deposits.

4.  Dental floss or interdental brushes should be 

used wherever possible.

5.  Toothpaste containing fluoride should be used 

in areas where natural fluoride is not present in 

the water supply. Fluoride supplements may also 

be prescribed if appropriate.

6.  An orthodontic assessment should be consid-

ered for all patients between the ages of 10–14 

in order to determine if there are any problems 

associated with overcrowding, which can result 

in periodontal disease if left untreated.

7.  Close liaison between the dental surgeon and 

the hemophilia team is essential to provide good 

comprehensive dental care.

8.  Treatment can be safely carried out under local 

anesthesia using the full range of techniques 

available to dental surgeons. Infiltration, intra-

papillary, and intra-ligamentary injections are 

often done under factor cover (20-40%) though 

it may be possible for those with adequate expe-

rience to administer these injections without 

it. (Level 4) [57,58]

9.  Treatment from the hemophilia unit may be 

required before an inferior alveolar nerve block 

or lingual infiltration. 

10. Dental extraction or surgical procedures carried 

out within the oral cavity should be done with 

a plan for hemostasis management, in consul-

tation with the hematologist. (Level 3) [51]

11. Tranexamic acid or epsilon aminocaproic acid 

(EACA) is often used after dental procedures 

to reduce the need for replacement therapy. 

(Level 4) [59,60]

12. Oral antibiotics should only be prescribed if clin-

ically necessary. 

13. Local hemostatic measures may also be used 

whenever possible following a dental extraction. 

Typical products include oxidized cellulose and 

fibrin glue.

14. Following a tooth extraction, the patient should 

be advised to avoid hot food and drinks until 

normal feeling has returned. Smoking should 

be avoided as this can cause problems with 

healing. Regular warm salt water mouthwashes (a 

teaspoon of salt in a glass of warm water) should 

begin the day after treatment and continue for 

five to seven days or until the mouth has healed. 

15. Prolonged bleeding and/or difficulty in speaking, 

swallowing, or breathing following dental manip-

ulation should be reported to the hematologist/

dental surgeon immediately.

GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA

18

16. Non-steroidal anti-inflammatory drugs (NSAIDs) 

and aspirin must be avoided.

17. An appropriate dose of paracetamol/acetamin-

ophen every six hours for two to three days will 

help prevent pain following an extraction.

18. The presence of blood-borne infections should 

not affect the availability of dental treatment. 

19. Prevention of bleeding at the time of dental proce-

dures in patients with inhibitors to FVIII or FIX 

requires careful planning [61]. 

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21

2.1  Carriers

1.  Hemophilia is an X-linked disorder that typically 

affects males, while females are carriers.

2.  Obligate carriers are:

 ■ daughters of a person with hemophilia 

 ■ mothers of one son with hemophilia and who 

have at least one other family member with 

hemophilia

 ■ mothers of one son with hemophilia and who 

have a family member who is a known carrier 

of the hemophilia gene 

 ■ mothers of two or more sons with hemophilia

3.  The expected mean clotting factor level in carriers 

of hemophilia is 50% of the levels found in the 

healthy population [1,2]. 

4.  Most carriers are asymptomatic.

5.  Carriers with clotting factor levels of 40-60% of 

normal may have an increased bleeding tendency [3]. 

6.  A few carriers may have clotting factor levels in 

the hemophilia range—mostly in the mild cate-

gory—but in rare instances, carriers can be in the 

moderate or severe range due to extreme lyoniza-

tion (see Table 1-1).

7.  Carriers with clotting factor levels in the hemo-

philia range may be symptomatic with bleeding 

manifestations commensurate with their degree 

of clotting factor deficiency, particularly during 

trauma and surgery [3]. 

8.  Menorrhagia and bleeding after medical interven-

tions are the most common manifestations among 

carriers with significantly low factor levels [3]. 

9.  Carriers with low clotting factor levels should be 

categorized as having hemophilia of appropriate 

severity and managed accordingly.

10. Birth control pills and antifibrinolytic agents are 

useful in controlling symptoms of menorrhagia. 

11. Levels of factor VIII increase significantly in preg-

nancy. Levels of factor IX, however, do not usually 

change significantly [4]. 

12. Immediate female relatives (mother, sisters, and